Long QT syndrome: Cellular basis and arrhythmia mechanism in LQT2

Craig T. January, Qiuming Gong, Zhengfeng Zhou

    Research output: Contribution to journalReview articlepeer-review

    96 Scopus citations

    Abstract

    HERG Channel Dysfunction in LQT2. LQT2 is one form of the congenital long QT syndrome. It results from mutations in the human ether-a-go-go-related gene (HERG), and more than 80 mutations, usually causing single amino acid substitutions in the HERG protein, are known. HERG encodes the ion channel pore-forming subunit protein for the rapidly activating delayed rectifier K+ channel (IKr) in the heart. This review summarizes current findings about mutations causing LQT2, the mechanisms by which mutations may cause the clinical phenotype of a reduction in IKr and a prolonged QT interval, and how this may be involved in the generation of ventricular arrhythmias.

    Original languageEnglish (US)
    Pages (from-to)1413-1418
    Number of pages6
    JournalJournal of cardiovascular electrophysiology
    Volume11
    Issue number12
    DOIs
    StatePublished - Jan 1 2000

    Keywords

    • Arrhythmia
    • Cardiac action potentials
    • HERG
    • Long QT syndrome
    • Potassium channels
    • Sudden death

    ASJC Scopus subject areas

    • Cardiology and Cardiovascular Medicine
    • Physiology (medical)

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